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Adult Onset Still’s Disease

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DOI: 10.4236/ijcm.2015.610095    2,443 Downloads   2,995 Views  

ABSTRACT

The adult onset Still’s disease is a rare affection characterised by occurrence of fever, arthralgia or arthritis and evanescent cutaneous eruption. Multiple other systemic lesions make the diagnosis more difficult. Several diagnostic criteria were formulated to confirm this disease. The physiopathology of the adult onset Still’s disease is not well elucidated. However, several studies based on new facts in physiopathology, improved the therapy of refractory forms for which the biotherapy was an interesting alternative. The TNF alpha receptor antagonists are efficient on systemic and articular manifestations of this disease and allow a corticosteroid’s saving. Tocilizumab (interleukin 6 receptor antagonist), and Anakinra (interleukin 1 receptor antagonist) are also new promising treatments for resistant forms.

Conflicts of Interest

The authors declare no conflicts of interest.

Cite this paper

Mahfoudhi, M. , Gorsane, I. , Shimi, R. , Turki, S. and Abdallah, T. (2015) Adult Onset Still’s Disease. International Journal of Clinical Medicine, 6, 716-724. doi: 10.4236/ijcm.2015.610095.

References

[1] Bywaters, E.G.L. (1971) Still’s Disease in the Adult. Annals of the Rheumatic Diseases, 30, 121-133.
http://dx.doi.org/10.1136/ard.30.2.121
[2] Pouchot, J., Sampalis, J.S., Beaudet, F., Carette, S., Décary, F., Salusinsky-Sternbach, M., et al. (1991) Adult Still’s Disease: Manifestations, Disease Course, and Outcome in 62 Patients. Medicine, 70, 118-136.
[3] Van de Putte, L.B. and Wouters, J.M. (1991) Adult-Onset Still’s Disease. Clinical Rheumatology, 5, 263-275.
http://dx.doi.org/10.1016/s0950-3579(05)80283-3
[4] Bagnari, V., Colina, M., Ciancio, G., Govoni, M. and Trotta, F. (2010) Adult-Onset Still’s Disease. Rheumatology International, 30, 855-862.
http://dx.doi.org/10.1007/s00296-009-1291-y
[5] Sugiura, T., Maeno, N., Kawaguchi, Y., Takei, S., Imanaka, H., Kawano, Y., et al. (2006) A Promoter Haplotype of the Interleukin-18 Gene Is Associated with Juvenile Idiopathic Arthritis in the Japanese Population. Arthritis Research & Therapy, 8, R60.
http://dx.doi.org/10.1186/ar1930
[6] Arlet, J.B., Boutin, L.E., Thi Huong, D., Pouchot, J. and Piette, J.C. (2005) Physiopathologie de la maladie de Still de l’adulte. La Revue de Médecine Interne, 26, 549-556.
http://dx.doi.org/10.1016/j.revmed.2004.11.021
[7] Fujii, T., Nojima, T., Yasuaka, H., Satoh, S., Nakamura, K., Kuwana, M., et al. (2001) Cytokine and Immunogenetic Profiles in Japanese Patients with Adult Still’s Disease. Association with Chronic Articular Disease. Journal of Rheumatology, 40, 1398-1404.
http://dx.doi.org/10.1093/rheumatology/40.12.1398
[8] Gerfaud-Valentin, M., Jamilloux, Y., Iwaz, J. and Sève, P. (2014) Adult-Onset Still’s Disease. Autoimmunity Reviews, 13, 708-722.
http://dx.doi.org/10.1016/j.autrev.2014.01.058
[9] Hoshino, T., Ohta, A., Yang, D., Kawamoto, M., Kikuchi, M., Inoue, Y., et al. (1998) Elevated Serum Interleukin 6, Interferon-Gamma, and Tumor Necrosis Factor-Alpha Levels in Patients with Adult Still’s Disease. Journal of Rheumatology, 35, 396-398.
[10] Scheinberg, M.A., Chapira, E., Fernandes, M.L. and Hubscher, O. (1996) Interleukine 6: A Possible Marker of Disease Activity in Adult Onset Still’s Disease. Clinical and Experimental Rheumatology, 14, 653-655.
[11] Maria, A.T., Le Quellec, A., Jorgensen, C., Touitou, I., Rivière, S. and Guilpain, P. (2014) Adult Onset Still’s Disease (AOSD) in the Era of Biologic Therapies: Dichotomous View for Cytokine and Clinical Expressions. Autoimmunity Reviews, 13, 1149-1159.
http://dx.doi.org/10.1016/j.autrev.2014.08.032
[12] Kawachima, M., Yamamura, M., Taniai, M., Yamamura, M., Taniai, M.,Yamauchi, H., et al. (2001) Levels of Interleukin-18 and Its Binding Inhibitors in the Blood Circulation of Patients with Adult-Still’s Disease. Arthritis & Rheumatism, 44, 550-560.
http://dx.doi.org/10.1002/1529-0131(200103)44:3<550::AID-ANR103>3.0.CO;2-5
[13] Kawagushi, Y., Terajima, H., Harigai, M., Hara, M. and Kamatani, N. (2001) Interleukin-18 as a Novel Diagnostic Marker and Indicator of Disease Severity in Adult-Onset Still’s Disease. Arthritis & Rheumatism, 44, 1716-1717.
http://dx.doi.org/10.1002/1529-0131(200107)44:7<1716::AID-ART298>3.0.CO;2-I
[14] Matsui, K., Tsuchida, T., Hiroishi, K., Tominaga, K., Hayashi, N., Hada, T., et al. (1999) High Serum Level of Macrophage-Colony Stimulating Factor (M-CSF) in Adult Still’s Disease. Rheumatology, 38, 477-478.
http://dx.doi.org/10.1093/rheumatology/38.5.477
[15] Choi, J.H., Suh, C.L., Lee, Y.M., Suh, Y.J., Lee, S.K., Kim, S.S., et al. (2003) Serum Cytokine Profiles in Patients with Adult Onset Still’s Disease. The Journal of Rheumatology, 30, 2422-2427.
[16] Andres, E., Kurtz, J.E., Perrin, A.E., Pfumio, F., Ruellan, A., Goichot, B., et al. (2003) Retrospective Monocentric Study of 17 Patients with Adult Still’s Disease, with Special Focus on Liver Abnormalities. Hepato-Gastroenterology, 50, 1992-1995.
[17] Min, J.K., Cho, C.S., Kim, H.Y. and Oh, E.J. (2003) Bone Marrow Findings in Patients with Adult Still’s Disease. Scandinavian Journal of Rheumatology, 32, 119-121.
http://dx.doi.org/10.1080/03009740310000148
[18] Zollner, R.C., Kerm, P., Steininger, H., Kalden, J.R. and Manger, B. (1997) Hyperferritinemia in Still Syndrome in the Adult and Reactive Hemophagocytic Syndrome. Medizinische Klinik, 92, 494-498.
http://dx.doi.org/10.1007/BF03044919
[19] Coffernils, M., Soupart, A., Pradier, O., Feremans, W., Neve, P. and Decaux, G. (1992) Hyperferritinemia in Adult Onset Still’s Disease and the Hemophagocytic Syndrome. The Journal of Rheumatology, 19, 1425-1427.
[20] Saiki, O., Uda, H., Nishimoto, N., Miwa, T., Mima, T., Ogawara, T., et al. (2004) Adult Still’s Disease Reflects a Th2 Rather than a Th1 Cytokine Profile. Clinical Immunology, 112, 120-125.
http://dx.doi.org/10.1016/j.clim.2004.03.023
[21] Hoshino, T., Ohta, A., Nakao, M., Ota, T., Inokuchi, T., Matsueda, S., et al. (1996) TCR γδ+ T Cells in Peripheral Blood of Patients with Adult Still’s Disease. The Journal of Rheumatology, 23, 124-129.
[22] Wulffraat, N.M., Rijkers, G.T., Elst, E., Brooimans, R. and Kuis, W. (2003) Reduced Perforin Expression in Systemic Juvenile Idiopathic Arthritis Is Restored by Autologous Stem-Cell Transplantation. Rheumatology, 42, 375-379.
http://dx.doi.org/10.1093/rheumatology/keg074
[23] Pouchot, J. and Vinceneux, P. (2004) La maladie de Still de l’adulte: Diagnostic, évolution et pronostic, pathogénie et traitement de la maladie de Still de l’adulte. La Presse Médicale, 33, 1019-1025.
http://dx.doi.org/10.1016/S0755-4982(04)98831-8
[24] Yamaguchi, M., Ohta, A., Tsunematsu, T., Kasukawa, R., Mizushima, Y., Kashiwagi, H., et al. (1992) Preliminary Criteria for Classification of Adult Still’s Disease. The Journal of Rheumatology, 19, 424-430.
[25] Van Reeth, C., Le Moel, G., Lasne, Y., Revenant, M.C., Agneray, J., Kahn, M.F., et al. (1994) Serum Ferritin and Isoferritins Are Tools for Diagnosis of Active Adult Still’s Disease. The Journal of Rheumatology, 21, 890-895.
[26] Fautrel, B., Le Moel, G., Saint-Marcoux, B., Taupin, P., Vignes, S., Rozenberg, S., et al. (2001) Diagnostic Value of Ferritin and Glycosylated Ferritin in Adult Onset Still’s Disease. The Journal of Rheumatology, 28, 322-329.
[27] Vignes, S., Le Moel, G., Fautrel, B., Wechsler, B., Godeau, P. and Piette, J.C. (2000) Percentage of Glycosylated Serum Ferritin Remains Low throughout the Course of Adult Onset Still’s Disease. Annals of the Rheumatic Diseases, 59, 347-350.
http://dx.doi.org/10.1136/ard.59.5.347
[28] Lambotte, O., Cacoub, P., Costedoat, N., Le Moel, G., Amoura, Z. and Piette, J.C. (2003) High Ferritin and Low Glycosylated Ferritin May Also Be a Marker of Excessive Macrophage Activation. The Journal of Rheumatology, 30, 1027-1028.
[29] Fautrel, B., Zing, E., Golmard, J.L., Le Moel, G., Bissery, A., Rioux, C., et al. (2002) Proposal for a New Set of Classification Criteria for Adult-Onset Still Disease. Medicine, 81, 194-200.
http://dx.doi.org/10.1097/00005792-200205000-00003
[30] Kojima, M., Nakamura, S., Miyawaki, S., Yashiro, K., Oyama, T. and Itoh, H. (2002) Lymph Node Lesion in Adult-Onset Still’s Disease Resembling Peripheral T-Cell Lymphoma: A Report of Three Cases. International Journal of Surgical Pathology, 10, 197-202.
http://dx.doi.org/10.1177/106689690201000305
[31] Efthimiou, P., Paik, P.K. and Bielory, L (2006) Diagnosis and Management of Adult Onset Still’s Disease. Annals of the Rheumatic Diseases, 65, 564-572.
http://dx.doi.org/10.1136/ard.2005.042143
[32] Bennett, A.N., Peterson, P., Sangle, S., Hangartner, R., Abbs, I.C., Hughes, G.R., et al. (2004) Adult Onset Still’s Disease and Collapsing Glomerulopathy: Successful Treatment with Intravenous Immunoglobulins and Mycophenolate Mofetil. Rheumatology, 43, 795-799.
http://dx.doi.org/10.1093/rheumatology/keh172
[33] Emad, Y., Ragab, Y. and El-Shaarawy, N. (2012) Lichen Planus in Association with Adult-Onset still’s Disease Successfully Treated with Mycophenolate Mofetil. The Journal of Rheumatology, 39, 1305-1306.
http://dx.doi.org/10.3899/jrheum.120032
[34] Permal, S., Wechsler, B., Cabane, J., Perrot, S., Blum, L. and Imbert, J.C. (1995) Traitement de la maladie de Still de l’adulte par immunoglobulines intraveineuses. La Revue de Médecine Interne, 16, 250-254.
http://dx.doi.org/10.1016/0248-8663(96)80703-X
[35] Vignes, S., Wechsler, B., Amoura, Z., Papo, T., Francès, C., Huong, D.L., et al. (1998) Intravenous Immunoglobulin in Adult Still’s Disease Refractory to Non-Steroidal Anti-Inflammatory Drugs. Clinical and Experimental Rheumatology, 16, 295-298.
[36] Kim, H.A., Sung, J.M. and Suh, C.H. (2012) Therapeutic Responses and Prognosis in Adult-Onset Still’s Disease. Rheumatology International, 32, 1291-1298.
http://dx.doi.org/10.1007/s00296-011-1801-6
[37] Neto, N.S., Waldrich, L., de Carvalho, J.F. and Pereira, R.M. (2009) Adult-Onset Still’s Disease with Pulmonary and Cardiac Involvement and Response to Intravenous Immunoglobulin. Acta Reumatológica Portuguesa, 34, 628-632.
[38] Kraetsch, H.G., Antoni, C., Kalden, J.R. and Manger, B. (2001) Successful Treatment of a Small Cohort of Patients with Adult Onset of Still’s Disease with Infliximab: First Experiences. Annals of the Rheumatic Diseases, 60, iii55-iii57.
[39] Husni, M.E., Maier, A.L., Mease, P.J., Overman, S.S., Fraser, P., Gravallese, E.M., et al. (2002) Etanercept in the Treatment of Adult Patients with Still’s Disease. Arthritis & Rheumatism, 46, 1171-1176.
http://dx.doi.org/10.1002/art.10231
[40] Dechant, C., Schauenberg, P., Antoni, C.E., Kraetsch, H.G., Kalden, J.R. and Manger, B. (2004) Longterm Outcome of TNF Blockade in Adult-Onset Still’s Disease. Deutsche Medizinische Wochenschrift, 129, 1308-1312.
http://dx.doi.org/10.1055/s-2004-826865
[41] Fautrel, B., Sibilia, J., Mariette, X. and Combe, B., the Club Rhumatismes et Inflammation (2005) Tumour Necrosis Factor Alpha Blocking Agents in Refractory Adult Still’s Disease: An Observational Study of 20 Cases. Annals of the Rheumatic Diseases, 64, 262-266.
http://dx.doi.org/10.1136/ard.2004.024026
[42] Iwamoto, M., Nara, H., Hirata, D., Minota, S., Nishimoto, N. and Yoshizaki, K. (2002) Humanized Monoclonal Anti-Interleukin-6 Receptor Antibody for Treatment of Intractable Adultonset Still’s Disease. Arthritis & Rheumatism, 46, 3388-3389.
http://dx.doi.org/10.1002/art.10620
[43] Puéchal, X., DeBandt, M., Berthelot, J.M., Breban, M., Dubost, J.J., Fain, O., et al. (2011) Tocilizumab in Refractory Adult Still’s Disease. Arthritis Care & Research, 63, 155-159.
http://dx.doi.org/10.1002/acr.20319
[44] Suematsu, R., Ohta, A., Matsuura, E., Takahashi, H., Fujii, T., Horiuchi, T., et al. (2012) Therapeutic Response of Patients with Adult Still’s Disease to Biologic Agents: Multicenter Results in Japan. Modern Rheumatology, 22, 712-719.
http://dx.doi.org/10.3109/s10165-011-0569-6
[45] Yoshida, Y., Sakamoto, M., Yokota, K., Sato, K. and Mimura, T. (2011) Tocilizumab Improved both Clinical and Laboratory Manifestations Except for Interleukin-18 in a Case of Multiple Drug-Resistant Adult-Onset Still’s Disease. Internal Medicine, 50, 1757-1760.
http://dx.doi.org/10.2169/internalmedicine.50.4771
[46] Fitzgerald, A.A., Leclercq, S.A., Yan, A., Homik, J.E. and Dinarello, C.A. (2005) Rapid Responses to Anakinra in Patients with Refractory Adult-Onset Still’s Disease. Arthritis & Rheumatism, 52, 1794-1803.
http://dx.doi.org/10.1002/art.21061
[47] Lequerré, T., Quartier, P., Rosellini, D., Alaoui, F., De Bandt, M., Mejjad, O., et al. (2008) Interleukin-1 Receptor Antagonist (Anakinra) Treatment in Patients with Systemic-Onset Juvenile Idiopathic Arthritis or Adult Onset Still Disease: Preliminary Experience in France. Annals of the Rheumatic Diseases, 67, 302-308.
http://dx.doi.org/10.1136/ard.2007.076034
[48] Laskari, K., Tzioufas, A.G. and Moutsopoulos, H.M. (2011) Efficacy and Long-Term Follow-Up of IL-1R Inhibitor Anakinra in Adults with Still’s Disease: A Case-Series Study. Arthritis Research & Therapy, 13, R91.
http://dx.doi.org/10.1186/ar3366
[49] Hong, D.S., Yang, Z.H., Han, S.Y., Liang, X.G., Ma, K.F. and Zhang, X.G. (2014) Interleukin 1 Inhibition with Anakinra in Adult-Onset Still Disease: A Meta-Analysis of Its Efficacy and Safety. Drug Design, Development and Therapy, 8, 2345-2357.
[50] Ahmadi-Simab, K., Lamprecht, P., Jankowiak, C. and Gross, W.L. (2006) Successful Treatment of Refractory Adult Onset Still’s Disease with Rituximab. Annals of the Rheumatic Diseases, 65, 1117-1118.
http://dx.doi.org/10.1136/ard.2005.047621
[51] Bartoloni, E., Alunno, A., Luccioli, F., Santoboni, G. and Gerli, R. (2009) Successful Treatment of Refractory Adult-Onset Still’s Disease with Anti-CD20 Monoclonal Antibody. Clinical and Experimental Rheumatology, 27, 888-889.
[52] Kadavath, S. and Efthimiou, P. (2015) Adult-Onset Still’s Disease—Pathogenesis, Clinical Manifestations, and New Treatment Options. Annals of Medicine, 47, 6-14.
http://dx.doi.org/10.3109/07853890.2014.971052
[53] Jamilloux, Y., Gerfaud-Valentin, M., Henry, T. and Sève, P. (2014) Treatment of Adult-Onset Still’s Disease: A Review. Journal of Therapeutics and Clinical Risk Management, 11, 33-43.
[54] Narula, N., Narula, T. and Abril, A. (2015) Seizing the Clinical Presentation in Adult Onset Still’s Disease. An Extensive Literature Review. Autoimmunity Reviews, 14, 472-477.
http://dx.doi.org/10.1016/j.autrev.2015.01.007

  
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